Extraskeletal Ewing's sarcomas (EES) are rare. Recently, Ewing's sarcoma of the bone, primitive neuroectodermal tumor (PNET), Askin tumor and EES have been included into the family of Ewing's tumors, due to the overlapping features relating to their clinico-pathological and cytogenetic appearance. We experienced a case of an EES arising from the duodenum in a 14-year-old girl who presented with hematemesis and epigastric discomfort. A duodenal biopsy specimen revealed the infiltration of small round cells and rich vasculatures, with immunohistochemical finding of MIC-2 (CD99) (+), vimentin (+), CD56 (NCAM) (+), LCA (-), T-cell (-), B-cell (-), CD43 (-) and CD68 (-). She was treated with several cycles of multiagent chemotherapy, and achieved an initial partial response, but rapid progression of tumor followed, so she was treated with surgical excision. This is the first case report of an EES arising from the duodenum in the literature.
Adolescent ; B-Lymphocytes ; Biopsy ; Cytogenetics ; Drug Therapy ; Duodenum* ; Female ; Hematemesis ; Humans ; Neuroectodermal Tumors, Primitive ; Sarcoma ; Sarcoma, Ewing* ; T-Lymphocytes ; Vimentin

PURPOSE: The purpose of our study was to evaluate the outcome of intensified induction therapy using the Vanderbilt regimen in patients with a poor prognosis non-Hodgkin's lymphoma (NHL). MATERIALS AND METHODS: We retrospectively analyzed the results of two pilot studies, which enrolled the patients aged 60 years or less, with a previously untreated NHL of intermediate grade on the Working formulation, having 2 or 3 adverse prognostic factors on the age- adjusted International Prognostic Index. Patients received an intensified induction, with the regimen described by the Vanderbilt group. RESULTS: Thirty-five patients were analyzed. After induction, 29 patients (83%) achieved more than partial response (PR): 22 (63%) complete response (CR) and 7 (20%) PR. Three of the PRs were subsequently converted to CR following consolidation therapy. The overall CR rate, following the completion of treatment, was 71%. The 3-year overall survival (OS) rate of all patients was 53%. In the univariate analysis, age (50 years) was the only factor affecting the OS. The 3-year disease-free survival (DFS) rate of patients with CR was 68%. In the univariate analysis, age and bone marrow involvement were the factors affecting the DFS. Two patients died from the treatment-related toxicity of the induction therapy: one due to sepsis and the other due to congestive heart failure. CONCLUSION: Although the CR rate was relatively high, the OS or DFS of patients with a poor prognosis NHL, who had received the intensified induction using the Vanderbilt regimen, were no different from those that had received the conventional chemotherapy, as reported by the International Prognostic Index Project. However, the OS or DFS in the young patient groups were encouraging. To test the hypothesized benefits of our approach in the young patient groups, a larger cohort of patients aged 50 years or less should be studied.
Bone Marrow ; Cohort Studies ; Disease-Free Survival ; Drug Therapy ; Heart Failure ; Humans ; Lymphoma, Non-Hodgkin* ; Pilot Projects ; Prognosis ; Retrospective Studies ; Sepsis

BACKGROUND: The purpose of this study was to evaluate the clinical features and treatment outcome of patients with primary non- Hodgkin's lymphoma (NHL) of bone. METHODS: A survey of 1,050 NHL patients who had been diagnosed at Korea Cancer Center Hospital between 1989 and 2001 identified 32 patients with NHL involving bone. Of these 32 patients, 22 patients were diagnosed as primary NHL of bone. The clinical data were available in 17 of the patients, and analyzed retrospectively. RESULTS: The male to female ratio was 3.3:1 and the median age was 31 years (range, 16- 52 years). The most common presenting symptom was bone pain of involved site (76.5%) and the most commonly involved site was tibia. Stages at diagnosis were as follows: stage I in 6 patients; stage II in 2 patients; stage III in 2 patients; stage IV in 7 patients. According to the REAL classification, the most common histologic subtype was diffuse large B cell lymphoma. Of evaluable 16 patients, 5 patients had B symptoms. The risk groups accord-ing to International Prognostic Index (IPI) were as follows: low risk in 9 patients, low-intermediate risk in 4 patients, high-intermediate risk in 1 patient, high risk in 2 patients, not evaluable in 1 patient. The first-line treatment modalities were as follows: combined radiation and chemotherapy in 8 patients (1 for palliative aim, 7 for curative aim); chemotherapy only in 4 patients; radiation only in 2 patients; surgery and chemotherapy in 1 patient. Objective response rate to treatment was 93.3% (95% confidence interval; 80.9~100%) and five-year survival rate was 57% (95% confidence interval; 30.5~83.4%). CONCLUSION: Most patients complained bone pain at involved site. The histologic subtype was diverse, but the most common subtype was diffuse large B cell lymphoma. The prognosis is similar to other non- Hodgkin's lymphomas.
Classification ; Diagnosis ; Drug Therapy ; Female ; Hodgkin Disease ; Humans ; Korea ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin* ; Male ; Prognosis ; Retrospective Studies ; Survival Rate ; Tibia ; Treatment Outcome